Background Patients with chronic lymphocytic leukemia (CLL) are at a higher risk for developing
cutaneous malignancies. A study conducted in the United States reported that patients
with CLL were eight times more likely to have skin cancer than patients without CLL,
particularly squamous cell carcinoma (SCC). Many reports suggest that in CLL, the
dysfunctional lymphocytes are unable to elicit an antitumor response, thereby contributing
to the increased incidence and clinical characteristics of SCC and other forms of
skin cancers in these patients. Studies have shown data suggesting that the immunosuppressive
effects of chemotherapeutic agents and irradiation used to treat CLL increase the
risk of skin cancer in these patients, while other studies suggest genetic abnormalities
to be the cause of association between CLL and skin cancers. Cutaneous SCC in CLL
tends to be multiple and aggressive, with a higher incidence of posttreatment recurrence
and spread.
Malignant otitis externa (MOE) also referred to as skull base osteomyelitis occurs
mainly in elderly patients with diabetes or in immunocompromised patients. It is often
initiated by Pseudomonas, while methicillin-resistant Staphylococcus aureus has also been identified as a cause. It is characterized by persistent and severe,
deep-seated otalgia, foul-smelling purulent otorrhea, and granulation tissue or exposed
bone in the ear canal. Varying degrees of conductive hearing loss may occur. In severe
cases, facial nerve paralysis, and even lower cranial nerve (IX, X, or XI) paralysis,
may ensue as this erosive, potentially life-threatening infection spreads along the
skull base. Treatment is with systemic and topical antibiotics, regular aural toilet/microsuction,
meticulous control of diabetes, and in more extensive cases, surgical debridement
may be required.
Objectives Presentation of an unusual case of an elderly diabetic patient with CLL associated
with multiple head and neck cutaneous SCCs, MOE, and facial nerve paralysis.
We discuss the challenges/dilemmas in managing this complex case including diagnosis,
decision for antibiotics and surgical intervention, and timings for chemotherapy and
radiotherapy.
To the best of our knowledge, this is the first case of its kind in English literature.
Methods Case note review.
Results A multidisciplinary team consisting of ENT surgeons, the skull base team, an oncologist,
hematologist, radiologist, microbiologist, and pathologist were involved in the management
of this patient. Patient had chemotherapy for CLL and long-term antibiotics and surgical
debridement for MOE. His facial nerve paralysis had not improved. He subsequently
had a relapse of CLL and treatment for his cutaneous SCCs had to be deferred. The
patient has unfortunate now passed away.
Conclusion It is always a challenge to manage such complex cases. Radiotherapy for the CLL would
have worsened the MOE, whereas treating the MOE would have meant withholding/delaying
radiotherapy for the cutaneous SCC. Therefore, a multidisciplinary approach is vital
for the treatment of such cases. One of the main differential diagnoses of MOE is
malignancy. Patient education, frequent assessment, and low threshold for biopsy of
new lesions along with assessment of acute problems alongside existing disease allow
the most effective and appropriate treatment regimen to be devised.
